Prolonged survival after splenectomy in Wiskott-Aldrich syndrome: a case report
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چکیده
منابع مشابه
Prolonged survival after splenectomy in Wiskott-Aldrich syndrome: a case report
Wiskott-Aldrich syndrome is a rare X-linked immunodeficiency disorder that is characterized by a variable clinical phenotype. Matched donor bone marrow transplantation is currently the only curative therapeutic option. We present the case of a 24-year-old male who was diagnosed at the age of seven with Wiskott-Aldrich syndrome. He did not respond to intravenous gammaglobulin and he experienced ...
متن کاملWiskott-Aldrich Syndrome (WAS): A Case Report in Mauritius and Review
Wiskott-Aldrich is an X-lined recessive disorder typically characterized by thrombocytopenia, eczema and recurrent infections. We report the four year treatment progress of a six year old boy who initially presented with vesicular lesions over the trunk, upper and lower extremities and face and blood tinged stools at the age of 2 weeks. From the family pedigree, there were two suspected cases t...
متن کاملIntravenous immunoglobulin, splenectomy, and antibiotic prophylaxis in Wiskott-Aldrich syndrome.
AIM To assess the results of supportive treatment with intravenous immunoglobulin (IVIG) and antibiotic prophylaxis in combination with splenectomy in patients with Wiskott-Aldrich syndrome. STUDY DESIGN Retrospective review of case records of 21 patients from March 1984 to February 1996. RESULTS Thrombocytopenia was cured in 14 of 15 patients who had splenectomy, but it recurred intermitte...
متن کاملAutoimmune Features of Wiskott-Aldrich Syndrome: A Case Report
Diffuse alveolar hemorrhage in a pediatric patient requires urgent and aggressive therapy. Here we report a young child with Wiskott-Aldrich syndrome and antiplatelet antibody manifesting as recurrent pulmonary hemorrhage due to pauci-immune capillaritis that was successfully treated with rituximab.
متن کامل[The Wiskott-Aldrich syndrome].
can occur, the observed improvement cannot necessarily b)e attributed to the transfer factor. However, in two patients repeated remissions consistently followed transfer factor administration on repeated occasions. This included freedom from infections, regression of splenomegaly, and clearing of eczema. An unexpected finding was a decrease in bleeding in 3 of the 10 patients who had bleeding. ...
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ژورنال
عنوان ژورنال: Italian Journal of Pediatrics
سال: 2011
ISSN: 1824-7288
DOI: 10.1186/1824-7288-37-42